British Journal of Neurosurgery (1992) 6 , 583-586
SHORT REPORT
Solitary cervical lymphoma presenting as a neurofibroma
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
RAJU KAPOOR,* TAMAS REVESZt & MICHAEL POWELLS Departments of *Clinical Neurology, tNeuropathology and $Surgical Neurology, National Hospital for Neurology and Neurosurgery, Queen Square and Maida Vale, London W C l N 3BG and London W9 lTL, UK
Abstract A patient is described who presented with cervical cord compression. The imaging and operative findings were typical of a neurofibroma of the right third cervical root. However, histological studies confirmed that the tumour was a B-cell lymphoma. Isolated spinal lymphomas can therefore occur and may present as nerve sheath turnours.
Key words: Lymphoma, neurojibroma, spinal cord compression.
Introduction
It is unusual but well recognized for lymphomas to present with involvement of the spinal cord.'-4 Such tumours can either grow extradually, causing cord compression alone or after vertebral collapse, intradurally with leptomeningeal infiltration or as intramedullary deposit^.^.^ These patterns of growth do not generally overlap. In contrast we now report a case of non-Hodgkin's lymphoma which presented with cord compression and which formed an isolated mass arising from a solitary nerve root with both intra- and extradural components, so mimicking the macroscopic features of a neurofibroma. Case report
The patient was a 72-year-old Caucasian woman. One month before admission she developed paraesthesiae in the right hand, spreading a few days later to affect the right leg. The leg and arm became numb and then weak. A week after her symptoms began, the
left arm and leg were also affected in a similar manner. There was no sphincter disturbance or neck pain, although she had a long history of rheumatoid arthritis treated only with nonsteroidal anti-inflammatory drugs. On general examination there was a widespread arthropathy affecting the small and large joints symmetrically. There was no spinal tenderness nor any paravertebral mass, lymphadenopathy, organomegaly or any cutaneous lesions. The mental state and cranial nerves were normal. The limbs were flaccid with severe, global weakness of the right arm and leg and moderate weakness on the left. Movements of the diaphragm were normal. The jaw jerk was absent and the tendon jerks brisk, more so on the right. Both plantar responses were extensor. There was a loss of pinprick sensation at the C5 level on the left with distal loss to pinprick in the right hand and foot. Position and vibration sense were lost in the knees and wrists bilaterally. The blood count and film, routine biochemistry, serum protein electrophoresis, B12 and
583
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
584
Raju Kapoor, Tamas Revesz & Michael Powell
FIG. 1. Computed myelography showing a series of 5 mm thick axial sections through the cervical region immediately after the myelogram. A lesion is demonstrated widening the intervertebral canal of the right third cervical nerve (arrow) and compressing the spinal cord (arrowhead).
folate levels and chest radiograph were normal. The erythrocyte sedimentation rate was 36 mm in the first hour. Myelography and CT (Fig. 1) showed a large, lobulated and mainly extradural mass compressing the spinal cord from the right. The mass had widened the intervertebral foramen of the third cervical nerve, but there was no involvement of the adjacent vertebrae or soft tissues. The lumbar cerebrospinal fluid contained 10 red blood cells and six white blood cells per mm3, protein 5.4 g/l, glucose 4.3 mmol/l (plasma glucose 5.1 mmol/l), and on cytology only mature lymphocytes were observed. A cervical laminectomy was performed sparing part of the left side of C2 but extending the decompression well laterally to the right side of C2, C3 and C4. This
demonstrated a partly intradural, partly extradural tumour which appeared to have arisen from the third cervical root and which had the typical macroscopic appearances of a neurofibroma. The tumour was easily separated from the spinal cord, and removed, following the tumour through the root exit foramen. Postoperatively there was only minor improvement in the patient’s neurological deficit: and 3 months later she died of bronchopneumonia. Post-mortem examination was not performed.
Histology Light microscopy revealed an obviously malignant tumour (Fig. 2). Many of the tumouI cells were pyknotic and others showed crush
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
Cervical fymphorna
585
FIG.2. (a) A pleomorphic malignant tumour with frequent mitotic figures (arrow). H&E,x 185. (b) The tumour cells show a positive membranous reaction on the leucocyte common antigen immunostain. X 85. A typical example is shown by the arrow.
artifact. However, in restricted areas there was well-preserved tumour tissue composed mainly of relatively large tumour cells of the lymphoid series. The tumour cells presented scanty cytoplasm and round or oval nuclei with one or more small nucleoli, frequently apposed to the nuclear membrane. A proportion of the tumour cells were smaller in size and their nuclei were more irregular in shape. There was a diffuse, abundant reticulin network of fine fibrils without concentric perivascular arrangement. The lymphoid nature and the B-cell origin ofthe tumour cells could be confirmed by the leukocyte common antigen and L26 immuno-stains, respectively. A histological diagnosis of 'malignant, non-Hodgkin lymphoma' was made.
Discussion Both the radiological and operative appearances in thiS case were those of a nerve sheath tumour expanding the intervertebral foramen of the third cervical nerve and compressing the spinal cord. The histological demonstration of a lymphoma of B-cell origin was therefore unexpected. From the point of view of its origin, it was also of interest that the tumour had both intra- and extradural components, since lymphoma deposits in either of these two sites do not generally extend into the other. Extradural lymphomas can present as isolated spinal masses, but they are not thought to arise de novo.'J Rather, they originate by direct
spread from lesions in adjacent lymph nodes or vertebrae, for which there was no evidence in the present case. Conversely, isolated involvement of spinal roots occurs in lymphomatous leptomeningitis,6 but these lesions characteristically affect several spinal levels and do not cause gross foramina1 enlargement. Therefore, the tumour described here had several features that were unusual for a lymphoma involving the spine. Rarely lymphomas have been encountered both intradurally and in the peripheral nerves,"q6 suggesting that growth has occurred between the two sites along nerve fibres, but given that there is already evidence of tumour dissemination in these cases, this interpretation remains tentative. In this respect, evidence of tumour dissemination, including abnormalities in the peripheral blood, peripheral lymphadenopathy, paravertebral masses or radiological involvement of contiguous vertebrae,'.' was absent in our patient. Thus it is possible that the findings in this patient show that in rare cases highly focal meningeal lymphomas can develop de novo and then extend extradurally, eventually to present in the same way as nerve sheath tumours.
Address fur correspondence: Dr R. Kapoor, University Department of Clinical Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London W C l N 3BG, UK.
586
Raju Kapoor, Tamas Revesz &Michael Powell
References
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
1 Grant JW, Kaech D, Jones DB. Spinal cord compression as the first presentation of lymphoma-a review of 15 cases. Histopathology 1986; 10:1191-202. 2 Haddad P, Thaell JF, Kiely JM, Harrison EG, Miller RH. Lymphoma of the spinal extradural space. Cancer 1976; 38:1862-6. 3 Henson RA, Ulrich H. Cancer and the nervous system. The neurological manifestations of malignant systemic disease. Oxford: Blackwell, 1982; 268-86.
4 Levitt LJ, Dawson DM, Rosenthal DS, Moloney WC. CNS involvement in non-Hodgkin’s lymphomas. Cancer 1980; 45545-52. 5 O’Neill BP, Illig JJ. Primary central nervous system lymphoma. Mayo Clin Proc 1989; 64:1005-20. 6 Griffin JW, Thompson RT, Mitchison MJ, de Kiewiet JC, Welland FH. Lymphomatous leptomeningitis. Am J Med 1971; 51:200-8. 7 Bucy PC, Jerva MJ. Primary epidural spinal lymphosarcoma. J Neurosurg 1962; 19:142-52.
SHORT REPORT
Solitary cervical lymphoma presenting as a neurofibroma
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
RAJU KAPOOR,* TAMAS REVESZt & MICHAEL POWELLS Departments of *Clinical Neurology, tNeuropathology and $Surgical Neurology, National Hospital for Neurology and Neurosurgery, Queen Square and Maida Vale, London W C l N 3BG and London W9 lTL, UK
Abstract A patient is described who presented with cervical cord compression. The imaging and operative findings were typical of a neurofibroma of the right third cervical root. However, histological studies confirmed that the tumour was a B-cell lymphoma. Isolated spinal lymphomas can therefore occur and may present as nerve sheath turnours.
Key words: Lymphoma, neurojibroma, spinal cord compression.
Introduction
It is unusual but well recognized for lymphomas to present with involvement of the spinal cord.'-4 Such tumours can either grow extradually, causing cord compression alone or after vertebral collapse, intradurally with leptomeningeal infiltration or as intramedullary deposit^.^.^ These patterns of growth do not generally overlap. In contrast we now report a case of non-Hodgkin's lymphoma which presented with cord compression and which formed an isolated mass arising from a solitary nerve root with both intra- and extradural components, so mimicking the macroscopic features of a neurofibroma. Case report
The patient was a 72-year-old Caucasian woman. One month before admission she developed paraesthesiae in the right hand, spreading a few days later to affect the right leg. The leg and arm became numb and then weak. A week after her symptoms began, the
left arm and leg were also affected in a similar manner. There was no sphincter disturbance or neck pain, although she had a long history of rheumatoid arthritis treated only with nonsteroidal anti-inflammatory drugs. On general examination there was a widespread arthropathy affecting the small and large joints symmetrically. There was no spinal tenderness nor any paravertebral mass, lymphadenopathy, organomegaly or any cutaneous lesions. The mental state and cranial nerves were normal. The limbs were flaccid with severe, global weakness of the right arm and leg and moderate weakness on the left. Movements of the diaphragm were normal. The jaw jerk was absent and the tendon jerks brisk, more so on the right. Both plantar responses were extensor. There was a loss of pinprick sensation at the C5 level on the left with distal loss to pinprick in the right hand and foot. Position and vibration sense were lost in the knees and wrists bilaterally. The blood count and film, routine biochemistry, serum protein electrophoresis, B12 and
583
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
584
Raju Kapoor, Tamas Revesz & Michael Powell
FIG. 1. Computed myelography showing a series of 5 mm thick axial sections through the cervical region immediately after the myelogram. A lesion is demonstrated widening the intervertebral canal of the right third cervical nerve (arrow) and compressing the spinal cord (arrowhead).
folate levels and chest radiograph were normal. The erythrocyte sedimentation rate was 36 mm in the first hour. Myelography and CT (Fig. 1) showed a large, lobulated and mainly extradural mass compressing the spinal cord from the right. The mass had widened the intervertebral foramen of the third cervical nerve, but there was no involvement of the adjacent vertebrae or soft tissues. The lumbar cerebrospinal fluid contained 10 red blood cells and six white blood cells per mm3, protein 5.4 g/l, glucose 4.3 mmol/l (plasma glucose 5.1 mmol/l), and on cytology only mature lymphocytes were observed. A cervical laminectomy was performed sparing part of the left side of C2 but extending the decompression well laterally to the right side of C2, C3 and C4. This
demonstrated a partly intradural, partly extradural tumour which appeared to have arisen from the third cervical root and which had the typical macroscopic appearances of a neurofibroma. The tumour was easily separated from the spinal cord, and removed, following the tumour through the root exit foramen. Postoperatively there was only minor improvement in the patient’s neurological deficit: and 3 months later she died of bronchopneumonia. Post-mortem examination was not performed.
Histology Light microscopy revealed an obviously malignant tumour (Fig. 2). Many of the tumouI cells were pyknotic and others showed crush
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
Cervical fymphorna
585
FIG.2. (a) A pleomorphic malignant tumour with frequent mitotic figures (arrow). H&E,x 185. (b) The tumour cells show a positive membranous reaction on the leucocyte common antigen immunostain. X 85. A typical example is shown by the arrow.
artifact. However, in restricted areas there was well-preserved tumour tissue composed mainly of relatively large tumour cells of the lymphoid series. The tumour cells presented scanty cytoplasm and round or oval nuclei with one or more small nucleoli, frequently apposed to the nuclear membrane. A proportion of the tumour cells were smaller in size and their nuclei were more irregular in shape. There was a diffuse, abundant reticulin network of fine fibrils without concentric perivascular arrangement. The lymphoid nature and the B-cell origin ofthe tumour cells could be confirmed by the leukocyte common antigen and L26 immuno-stains, respectively. A histological diagnosis of 'malignant, non-Hodgkin lymphoma' was made.
Discussion Both the radiological and operative appearances in thiS case were those of a nerve sheath tumour expanding the intervertebral foramen of the third cervical nerve and compressing the spinal cord. The histological demonstration of a lymphoma of B-cell origin was therefore unexpected. From the point of view of its origin, it was also of interest that the tumour had both intra- and extradural components, since lymphoma deposits in either of these two sites do not generally extend into the other. Extradural lymphomas can present as isolated spinal masses, but they are not thought to arise de novo.'J Rather, they originate by direct
spread from lesions in adjacent lymph nodes or vertebrae, for which there was no evidence in the present case. Conversely, isolated involvement of spinal roots occurs in lymphomatous leptomeningitis,6 but these lesions characteristically affect several spinal levels and do not cause gross foramina1 enlargement. Therefore, the tumour described here had several features that were unusual for a lymphoma involving the spine. Rarely lymphomas have been encountered both intradurally and in the peripheral nerves,"q6 suggesting that growth has occurred between the two sites along nerve fibres, but given that there is already evidence of tumour dissemination in these cases, this interpretation remains tentative. In this respect, evidence of tumour dissemination, including abnormalities in the peripheral blood, peripheral lymphadenopathy, paravertebral masses or radiological involvement of contiguous vertebrae,'.' was absent in our patient. Thus it is possible that the findings in this patient show that in rare cases highly focal meningeal lymphomas can develop de novo and then extend extradurally, eventually to present in the same way as nerve sheath tumours.
Address fur correspondence: Dr R. Kapoor, University Department of Clinical Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London W C l N 3BG, UK.
586
Raju Kapoor, Tamas Revesz &Michael Powell
References
Br J Neurosurg Downloaded from informahealthcare.com by Nyu Medical Center on 01/07/15 For personal use only.
1 Grant JW, Kaech D, Jones DB. Spinal cord compression as the first presentation of lymphoma-a review of 15 cases. Histopathology 1986; 10:1191-202. 2 Haddad P, Thaell JF, Kiely JM, Harrison EG, Miller RH. Lymphoma of the spinal extradural space. Cancer 1976; 38:1862-6. 3 Henson RA, Ulrich H. Cancer and the nervous system. The neurological manifestations of malignant systemic disease. Oxford: Blackwell, 1982; 268-86.
4 Levitt LJ, Dawson DM, Rosenthal DS, Moloney WC. CNS involvement in non-Hodgkin’s lymphomas. Cancer 1980; 45545-52. 5 O’Neill BP, Illig JJ. Primary central nervous system lymphoma. Mayo Clin Proc 1989; 64:1005-20. 6 Griffin JW, Thompson RT, Mitchison MJ, de Kiewiet JC, Welland FH. Lymphomatous leptomeningitis. Am J Med 1971; 51:200-8. 7 Bucy PC, Jerva MJ. Primary epidural spinal lymphosarcoma. J Neurosurg 1962; 19:142-52.
